inflammatory vasculitits Flashcards
what is giant cell arteritis?
is the granulomatous inflammation of large cerebral arteries with occurs associated with PMR (poly myalgia rheumatic)
who does giant cell arteritis usually affect?
white females >50 yrs
what are 2 types of large vessel vasculitis?
- Takayasu arteritis
- Giant cell arteritis
what are the symptoms of Giant Cell Arteritis (GCA)?
- headache (main one)
- severe unilateral headache may be noticed when brushing hair
- jaw claudication
- blurred or double vision
- irreversible painless complete sight loss can occur rapidly
systemic symptoms:
- fever
- muscle aches
- weight loss
- loss of appetite
- peripheral oedema
how is giant cell arteritis diagnosed?
- clinical presentation
- raised ESR: usually 50 mm/hour or more
- temporal artery biopsy
what is found in a temporal artery biopsy for giant cell artiritis?
mono nucleated infiltration or granulomatous inflammation usually with multinucleate giant cell
what is the medication for GCA?
immediately start 40-60mg of prednisolone- DONT WAIT FOR BIOPSY!
then adjust depending on visual symptoms:
40mg- no visual symptoms
60mg- visual symptoms
aspirin
what is takayasu arteritis?
-granulomatous inflammation of the aorta and its major branches
who does takayasu arteritis normally affect?
<40
females
asians
what is polymyalgia rheumatica?
Chronic inflammatory, systemic condition that affects individuals (>50), associated with finding a giant cell arteritis on temporal artery biopsy
who does polymyalgia rheumatica typically affect?
> 50 years
patients with GCA
how does polymyalgia rheumatica present?
- pain and stiffness in the shoulder and hip girdle
- sudden onset
- usually symmetrical
- worse in morning
what are the investigations for polymyalgia rheumatica?
- Raised ESR, plasma viscosity, CRP
- temporal artery biopsy
- temporal artery USS
what is the treatment for PMR?
-start at prednisolone 15 mg daily
what are the three types of ANCA-associated small cell vasculitis?
- GPA (granulomatous with polyangitis)
- MPA (microscopic polyangitis)
- EGPA (eosonophiliic granulomatous with polyangitis)
what was GPA previously called?
Wegener’s
what was EGPA previously called?
Churg-Strauss syndrome
what does EGPA often cause?
- often lung problems (asthma)
- often skin problems
- less often kidneys
what does GPA cause?
ENT problems:
- nose bleeds
- sinusitis
- hearing loss
-glomerulonephritis
what does MPA cause?
- renal failure
- haemoptysis
what is pANCA associated with?
MPA and EGPA
what is cANCA and PR3 associated with?
GPA
what investigations are done for ANCA associated small vessel vasculitis?
- immunofloresence to detect the ANCA (eg if it is cANCA or pANCA)
- inflammatory markers
- U and Es
- urinalysis for renal vasculitis
- CXR
- biopsy of affected area e.g. skin or kidney
what is the treatment for TA (takayasu arteritis)?
1st= Steroid sparing agents such as Leflynomide and methotrexate
2nd (if disease doesnt go away after a couple years)= Tocilizumab (IL-6 blocking agent)
